NICE Pathways bring together all NICE guidance, quality standards and other NICE information on a specific topic.

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1 bring together all NICE guidance, quality standards and other NICE information on a specific topic. are interactive and designed to be used online. They are updated regularly as new NICE guidance is published. To view the latest version of this pathway see: Pathway last updated: 12 October 2016 This document contains a single pathway diagram and uses numbering to link the boxes to the associated recommendations. All rights reserved

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3 1 Person over 16 with suspected or previously diagnosed COPD No additional information 2 Symptoms A diagnosis of COPD should be considered in patients over the age of 35 who have a risk factor (generally smoking) and who present with one or more of the following symptoms: exertional breathlessness chronic cough regular sputum production frequent winter 'bronchitis' wheeze. Patients in whom a diagnosis of COPD is considered should also be asked about the presence of the following factors: weight loss effort intolerance waking at night ankle swelling fatigue occupational hazards chest pain haemoptysis. NB These last two symptoms are uncommon in COPD and raise the possibility of alternative diagnoses. NICE has produced a pathway on suspected cancer recognition and referral. Breathlessness One of the primary symptoms of COPD is breathlessness. The Medical Research Council dyspnoea scale [See page 12] should be used to grade the breathlessness according to the level of exertion required to elicit it. Page 3 of 21

4 Patients should be regularly asked about their ability to undertake activities of daily living and how breathless they become when doing these. Anxiety and depression Healthcare professionals should be alert to the presence of depression in patients with COPD. The presence of anxiety and depression should be considered in patients: who are hypoxic who have severe dyspnoea who have been seen at or admitted to a hospital with an exacerbation of COPD. See the NICE pathway on depression, which includes recommendations on depression in adults with a chronic physical health problem, and the NICE pathway on anxiety. Quality standards The following quality statement is relevant to this part of the pathway. Chronic obstructive pulmonary disease in adults quality standard 1. Diagnosis with spirometry 3 Spirometry Spirometry should be performed in patients who are over 35, current or ex-smokers, and have a chronic cough. Spirometry should be considered in patients with chronic bronchitis. A significant proportion of these will go on to develop airflow limitation 1. Spirometry should be performed: at the time of diagnosis to reconsider the diagnosis, if patients show an exceptionally good response to treatment. Measure post-bronchodilator spirometry to confirm the diagnosis of COPD. Consider alternative diagnoses or investigations in: older people without typical symptoms of COPD where the FEV 1 /FVC ratio is < 0.7 Page 4 of 21

5 1 Celli BR, MacNee W. (2004) Standards for the diagnosis and treatment of patients with COPD: a summary of the ATS/ERS position paper. European Respiratory Journal 23(6): Page 5 of 21

6 younger people with symptoms of COPD where the FEV 1 /FVC ratio is 0.7. It is recommended that ERS 1993 reference values 1 are used but it is recognised that these values may lead to under-diagnosis in older people and are not applicable in black and Asian populations 2. In most patients routine spirometric reversibility testing is not necessary as a part of the diagnostic process or to plan initial therapy with bronchodilators or corticosteroids. It may be unhelpful or misleading because: repeated FEV 1 measurements can show small spontaneous fluctuations the results of a reversibility test performed on different occasions can be inconsistent and not reproducible over-reliance on a single reversibility test may be misleading unless the change in FEV 1 is greater than 400 ml the definition of the magnitude of a significant change is purely arbitrary response to long-term therapy is not predicted by acute reversibility testing. Quality standards The following quality statement is relevant to this part of the pathway. Chronic obstructive pulmonary disease in adults quality standard 1. Diagnosis with spirometry 4 Additional investigations At the time of their initial diagnostic evaluation in addition to spirometry all patients should have: a chest radiograph to exclude other pathologies a full blood count to identify anaemia or polycythaemia BMI calculated. Additional investigations [See page 14] should be performed to aid management in some circumstances. BMI should be calculated in patients with COPD: the normal range for BMI is 20 to less than 25 (not reviewed as part of the 2010 guideline update; NICE guideline CG43 (2006) on obesity states a healthy range is 18.5 to 24.9 kg/ Page 6 of 21

7 1 Quanjer PH, Tammeling GJ, Cotes JE et al. (1993) Lung volumes and forced ventilatory flows. Report Working Party Standardization of Lung Function Tests, European Community for Steel and Coal. Official Statement of the European Respiratory Society. European Respiratory Journal (Suppl) 16: Definitive spirometry reference values are not currently available for all ethnic populations. The GDG was aware of ongoing research in this area. Page 7 of 21

8 m 2, but this may not be appropriate for people with COPD). In older patients attention should also be paid to changes in weight, particularly if the change is more than 3 kg. Clinicians involved in the care of people with COPD should assess their need for occupational therapy using validated tools. 5 Severity assessment Be aware that disability in COPD can be poorly reflected in the FEV 1. A more comprehensive assessment of severity includes the degree of airflow obstruction and disability, the frequency of exacerbations and the following known prognostic factors: FEV 1 T L CO breathlessness (MRC scale) health status exercise capacity (for example, 6-minute walk test) BMI partial pressure of oxygen in arterial blood (PaO 2 ) cor pulmonale. Calculate the BODE index to assess prognosis where its component information is currently available. Severity of airflow obstruction [See page 12] should be assessed according to the reduction in FEV 1. 6 Differentiating between COPD and asthma COPD and asthma are frequently distinguishable on the basis of history (and examination) in untreated patients presenting for the first time. Features from the history and examination should be used to differentiate COPD from asthma whenever possible (see clinical features differentiating COPD and asthma [See page 15]). Longitudinal observation of patients (whether using spirometry, peak flow or symptoms) should also be used to help differentiate COPD from asthma. Page 8 of 21

9 To help resolve cases where diagnostic doubt remains, or both COPD and asthma are present, the following findings should be used to help identify asthma: a large (> 400 ml) response to bronchodilators a large (> 400 ml) response to 30 mg oral prednisolone daily for 2 weeks serial peak flow measurements showing 20% or greater diurnal or day-to-day variability. Clinically significant COPD is not present if the FEV 1 and FEV 1 /FVC ratio return to normal with drug therapy. If diagnostic uncertainty remains, referral for more detailed investigations, including imaging and measurement of T L CO, should be considered. If patients report a marked improvement in symptoms in response to inhaled therapy, the diagnosis of COPD should be reconsidered. For further information on diagnosing asthma, see the NICE pathway on asthma. 7 When to refer It is recommended that referrals for specialist advice are made when clinically indicated. Referral may be appropriate at all stages of the disease and not solely in the most severely disabled patients (see reasons for referral [See page 16]). Patients who are referred do not always have to be seen by a respiratory physician. In some cases they may be seen by members of the COPD team who have appropriate training and expertise. Patients identified as having alpha-1 antitrypsin deficiency should be offered the opportunity to be referred to a specialist centre to discuss the clinical management of this condition. BMI should be calculated in patients with COPD: If the BMI is abnormal (high or low), or changing over time, the patient should be referred for dietetic advice. Patients disabled by COPD should be considered for referral for assessment by a social services department. Page 9 of 21

10 8 Training and skills All health professionals involved in the care of people with COPD should have access to spirometry and be competent in the interpretation of the results. Spirometry can be performed by any healthcare worker who has undergone appropriate training and who keeps his or her skills up to date. Spirometry services should be supported by quality control processes. Quality standards The following quality statement is relevant to this part of the pathway. Chronic obstructive pulmonary disease in adults quality standard 1. Diagnosis with spirometry 9 Managing co-existing conditions Alpha-1 antitrypsin deficiency Alpha-1 antitrypsin replacement therapy is not recommended for patients with alpha-1 antitrypsin deficiency (see also when to refer [See page 9] in this pathway). Cor pulmonale A diagnosis of cor pulmonale should be considered if patients have: peripheral oedema a raised venous pressure a systolic parasternal heave a loud pulmonary second heart sound. It is recommended that the diagnosis of cor pulmonale is made clinically and that this process should involve excluding other causes of peripheral oedema. Patients presenting with cor pulmonale should be assessed for the need for LTOT. Page 10 of 21

11 Oedema associated with cor pulmonale can usually be controlled symptomatically with diuretic therapy. The following are not recommended for the treatment of cor pulmonale: angiotensin-converting enzyme inhibitors calcium channel blockers alpha-blockers digoxin (unless there is atrial fibrillation). Low body mass index BMI should be calculated in patients with COPD: If the BMI is low patients should also be given nutritional supplements to increase their total calorific intake and be encouraged to take exercise to augment the effects of nutritional supplementation. For information on nutrition support in adults who are malnourished or at risk of malnutrition see the NICE pathway on nutrition support in adults. For information on weight loss see the NICE pathway on obesity. Multiple long-term conditions NICE has produced a pathway on multimorbidity. 10 Managing exacerbations See Chronic obstructive pulmonary disease / Managing exacerbations of COPD 11 Managing stable COPD See Chronic obstructive pulmonary disease / Managing stable COPD Page 11 of 21

12 Severity of airflow obstruction Severity of airflow obstruction NICE clinical guideline 12(2004) ATS/ERS GOLD NICE clinical guideline 101(2010) FEV 1 /FVC FEV 1 % predicted Postbronchodilator Postbronchodilator Postbronchodilator Postbronchodilator < % Mild Stage 1 Mild Stage 1 Mild 3 < % Mild Moderate Stage 2 Moderate Stage 2 Moderate < % Moderate Severe Stage 3 Severe Stage 3 Severe < 0.7 < 30% Severe Very severe Stage 4 Very severe 4 Stage 4 Very severe Medical Research Council dyspnoea scale Grade Degree of breathlessness related to activities 1 Not troubled by breathlessness except on strenuous exercise Page 12 of 21

13 1 Celli BR, MacNee W (2004) Standards for the diagnosis and treatment of patients with COPD: a summary of the ATS/ERS position paper. European Respiratory Journal 23(6): GOLD (2008) Global strategy for the diagnosis, management, and prevention of chronic obstructive pulmonary disease. 3 Symptoms should be present to diagnose COPD in people with mild airflow obstruction. 4 Or FEV 1 < 50% with respiratory failure. Page 13 of 21

14 2 Short of breath when hurrying or walking up a slight hill 3 Walks slower than contemporaries on level ground because of breathlessness, or has to stop for breath when walking at own pace 4 Stops for breath after walking about 100 metres or after a few minutes on level ground 5 Too breathless to leave the house, or breathless when dressing or undressing Adapted from Fletcher CM, Elmes PC, Fairbairn MB et al. (1959) The significance of respiratory symptoms and the diagnosis of chronic bronchitis in a working population. British Medical Journal 2: Additional investigations Investigation Role Serial domiciliary peak flow measurements To exclude asthma if diagnostic doubt remains Alpha-1 antitrypsin If early onset, minimal smoking history or family history T L CO To investigate symptoms that seem disproportionate to the spirometric impairment To investigate symptoms that seem disproportionate to the spirometric impairment CT scan of the thorax To investigate abnormalities seen on a chest radiograph To assess suitability for surgery Page 14 of 21

15 ECG To assess cardiac status if features of cor pulmonale Echocardiogram To assess cardiac status if features of cor pulmonale Pulse oximetry To assess need for oxygen therapy if cyanosis or cor pulmonale present, or if FEV 1 <50% predicted Sputum culture To identify organisms if sputum is persistently present and purulent Clinical features differentiating COPD and asthma COPD Asthma Smoker or ex-smoker Nearly all Possibly Symptoms under age 35 Rare Often Chronic productive cough Common Uncommon Breathlessness Persistent and progressive Variable Night time waking with breathlessness and/or wheeze Uncommon Common Significant diurnal or day-to-day variability to day-to-day variability of symptoms Uncommon Common Page 15 of 21

16 Reasons for referral Reason Purpose There is diagnostic uncertainty Confirm diagnosis and optimise therapy Suspected severe COPD Confirm diagnosis and optimise therapy The patient requests a second opinion Confirm diagnosis and optimise therapy Onset of cor pulmonale Confirm diagnosis and optimise therapy Assessment for oxygen therapy Optimise therapy and measure blood gases Assessment for long-term nebuliser therapy Optimise therapy and exclude inappropriate prescriptions Assessment for oral corticosteroid therapy Justify need for long-term treatment or supervise withdrawal Bullous lung disease Identify candidates for surgery A rapid decline in FEV 1 Encourage early intervention Assessment for pulmonary rehabilitation Identify candidates for pulmonary rehabilitation Assessment for lung volume reduction surgery Identify candidates for surgery Assessment for lung transplantation Identify candidates for surgery Page 16 of 21

17 Dysfunctional breathing Confirm diagnosis, optimise pharmacotherapy and access other therapists Onset of symptoms under 40 years or a family history of alpha-1 antitrypsin deficiency Identify alpha-1 antitrypsin deficiency, consider therapy and screen family Uncertain diagnosis Make a diagnosis Symptoms disproportionate to lung function deficit Look for other explanations including cardiac impairment, pulmonary hypertension, depression and hyperventilation Frequent infections Exclude bronchiectasis Haemoptysis Exclude carcinoma of the bronchus Glossary ASA American Society of Anesthesiologists ATS American Thoracic Society BODE body mass index, airflow obstruction, dyspnoea and exercise capacity BTS British Thoracic Society Page 17 of 21

18 CEN Comité European de Normalisation (European Committee for Standardisation) COPD chronic obstructive pulmonary disease cor pulmonale in the context of this pathway, the term 'cor pulmonale' has been adopted to define a clinical condition that is identified and managed on the basis of clinical features. This clinical syndrome of cor pulmonale includes patients who have right heart failure secondary to lung disease and those in whom the primary pathology is retention of salt and water, leading to the development of peripheral oedema ECG electrocardiogram ERS European Respiratory Society FEV1 forced expiratory volume in 1 second FVC forced vital capacity GOLD global initiative for chronic obstructive lung disease ICS inhaled corticosteroid Page 18 of 21

19 LABA long-acting beta 2 agonist LAMA long-acting muscarinic antagonist LTOT long-term oxygen therapy MRC Medical Research Council NIV non-invasive ventilation PaO2 partial pressure of oxygen in arterial blood PaCO2 partial pressure of carbon dioxide in arterial blood PEF peak expiratory flow SABA short-acting beta 2 agonist SAMA short-acting muscarinic antagonist Page 19 of 21

20 SaO2 oxygen saturation of arterial blood TLCO carbon monoxide lung transfer factor Sources Chronic obstructive pulmonary disease (2010) NICE guideline CG101 Your responsibility The guidance in this pathway represents the view of NICE, which was arrived at after careful consideration of the evidence available. Those working in the NHS, local authorities, the wider public, voluntary and community sectors and the private sector should take it into account when carrying out their professional, managerial or voluntary duties. Implementation of this guidance is the responsibility of local commissioners and/or providers. Commissioners and providers are reminded that it is their responsibility to implement the guidance, in their local context, in light of their duties to avoid unlawful discrimination and to have regard to promoting equality of opportunity. Nothing in this guidance should be interpreted in a way which would be inconsistent with compliance with those duties. Copyright Copyright National Institute for Health and Care Excellence All rights reserved. NICE copyright material can be downloaded for private research and study, and may be reproduced for educational and not-for-profit purposes. No reproduction by or for commercial organisations, or for commercial purposes, is allowed without the written permission of NICE. Contact NICE National Institute for Health and Care Excellence Level 1A, City Tower Piccadilly Plaza Manchester M1 4BT Page 20 of 21

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